Sickle Cell Trait and Renal Disease

Abstract

I ndividuals with sickle cell trait (SCT), the heterozygous state of sickle hemoglobin β-globin gene (HbAS), are generally reassured that their health will not be affected by their carrier status. Renal disease, especially hematuria, is one of the most common and severe complications experienced by patients with sickle cell disease (SCD); but a complete understanding of the relationship between SCT and the development of chronic kidney disease (CKD) is still lacking. In this short review, we present an overview of SCT and renal complications in SCT, and discuss and identify SCT as a risk factor resulting from an interplay between genetic and environmental influences. Although SCT itself may not be a disease in itself, there is evidence suggesting clinical conditions related to SCT. Additionally, we highlight the rationale for further studies into this area, which could affect the global public health recommendations on any associated health risks.

Renal complications of sickle cell disease

“…Hematuria in SCD patients is due to micro-infarction in the renal papilla, which is often self-limiting but may lead to renal necrosis and clot formation (1,5). Hematuria often occurs at an older age and there is no difference in the incidence of both genders (7).…”

“…The inability to increase urinary concentration to 450 mmol/kg is a common finding in SCD patients (5). Urine concentrating ability in SCD patients is associated with the deletion of the α-globin gene; deletion of two genes reduces urine concentration (1).…”

“…The β-globin gene is located on the short arm of chromosome 11. Additionally, the sickle cell trait is caused by inheriting a copy of the mutation (1). Hemoglobin SS (HbSS) disease is the most common type of SCD and approximately one-third of SCD patients are heterozygous for hemoglobin SD (HbSD) (2).…”